Pediatric vertebral and spinal epidural tumors: a retrospective review of twelve cases.

OBJECTIVE: Pediatric vertebral tumors are rare, and most of the reported series have limited numbers of cases. Diagnosis of these tumors is difficult because of the patients' age and the rarity of the lesions. We aimed to report the clinical, radiological and pathological characteristics in a small series of pediatric vertebral and spinal epidural tumors and to discuss diagnostic and treatment difficulties. MATERIALS AND METHODS: Twelve consecutive pediatric cases with vertebral or spinal epidural tumors were reviewed retrospectively. RESULTS: The mean age was 12.6 years, and male and female patients were equal in number. The most common symptom was pain. There were some neurological or local findings in all patients, and there were some positive results on plain radiographs in all cases except 2. The tumors were removed totally in 9 cases. There were histologically malignant lesions in 3 and benign lesions in 9 cases. Three patients with malignant tumors were treated by radiotherapy and 2 by chemotherapy. One patient with thoracic hemangioma was also treated by embolization after surgery. One case with cervical Dabska's tumor died due to air embolization. The other patients were followed for 48.3 months. The preoperative neurological deficits were resolved completely in all patients except the one with only biopsy performed at the last follow-up. There were no new spinal deformities on follow-up. CONCLUSION: Children with vertebral tumors and spinal epidural tumors usually present with pain and neurological deficits or local findings, and there are some indications on plain radiographs. Therefore, a careful physical examination and detailed evaluation of radiographs may minimize the rate of misdiagnosis and underestimation. Most of these tumors are benign, therefore, their outcome is good, and cure may be possible for many of the cases.

Lumbar extradural infiltrating angiolipoma: a case report and review of 17 previously reported cases with infiltrating spinal angiolipomas.

BACKGROUND CONTEXT: Spinal extradural angiolipomas are rare benign tumors, and most of them are noninfiltrating tumors located in the extradural space. However, there are 17 cases with extradural infiltrating spinal angiolipomas extending into the vertebral bodies or posterior vertebral arches in literature. These 17 tumors are mostly located at the thoracic region, and anterior or in the anterolateral extradural space, and they generally infiltrate only one vertebra. Only two of them are located at the lumbar region, four are mainly located in the posterior extradural space, and three infiltrate more than one vertebra. PURPOSE: To present an exceptional case with infiltrating extradural spinal angiolipoma involving two lumbar segments and mainly located in the posterior extradural space. STUDY DESIGN: A case report. METHODS: A 41-year-old woman with infiltrating spinal angiolipoma was treated by incomplete surgical removal of the tumor. RESULTS: There were no complaints nor recurrence after 18 months follow-up. CONCLUSIONS: Outcome after surgery for spinal angiolipomas is very good overall even in the cases with infiltrating tumors. Although complete removal is certainly preferred, outcomes remained favorable despite incomplete resections.

Posterior fossa dermoid cysts causing cerebellar abscesses.

Dermoid cysts are uncommon tumors, and posterior fossa dermoid cysts may rarely cause abscess formation or formation of daughter abscesses within the cerebellum. At present, there are only 16 cases with posterior fossa dermoid cysts causing cerebellar abscesses reported in the literature. Two cases, 22 and 14 months old, with posterior fossa dermoid cysts and dermal sinus causing multiple cerebellar abscesses are reported. In the first one, there was also marked hydrocephalus. Retrospective examination of the patients revealed pinpoint-sized dimples in their suboccipital regions. Both patients were treated with antibiotics and underwent posterior fossa surgery. In the patient with marked hydrocephalus, ventriculoperitoneal shunting was performed after treatment of the infection. Both patients were neurologically normal, and there were no complaints, except a light learning difficulty in the patient with ventriculoperitoneal shunting, 133 and 34 months after surgery, respectively. Early detection of congenital dermal abnormalities along the craniospinal axis by routine examination of newborns is highly important before development of serious complications. Because surgery is the only effective treatment modality for these lesions, radical excision should be performed in all cases to avoid tumor recurrence. However, subtotal excision may be performed in selected cases, because the cyst capsule may adhere firmly to vital structures.

Cervical spinal brucellosis causing epidural and prevertebral abscesses and spinal cord compression: a case report.

BACKGROUND CONTEXT: Cervical involvement due to spinal brucellosis is quite rare. Although surgery usually is not necessary in spinal brucellosis, most of the patients with cervical involvement require surgical treatment because of the high rate of neurological involvement and spinal cord compression. PURPOSE: To present a unique case with cervical spinal brucellosis with epidural and paravertebral abscesses and to discuss the treatment alternatives of this disease. STUDY DESIGN: A case report. METHODS: A 61-year-old patient with spinal cord compression syndrome due to cervical spinal brucellosis was reported. He was treated by triplet antibiotherapy for 24 weeks. On magnetic resonance imaging, spinal cord compression caused by epidural abscess and granulation tissue, and prevertebral abscess were seen. RESULTS: At the end of the treatment, there were no complaints, neurological findings, or positive infection markers. There was not epidural compression on control magnetic resonance imaging. CONCLUSIONS: Surgery may not be required in all cervical spinal brucellosis cases with epidural compression and neurological involvement. Conservative treatment with close observation may be sufficient in these patients who are usually older people.

Non-contiguous multifocal spinal tuberculosis involving cervical, thoracic, lumbar and sacral segments: a case report.

MAIN PROBLEM: There are only a few cases reported with non-contiguous spinal tuberculosis in the literature. Most of these patients have only two non-contiguous lesions, and in almost all of these cases, surgical treatment is required. METHODS: A 17-year-old girl with non-contiguous multifocal spinal tuberculosis involving cervical, thoracic, thoracolumbar, lumbar and sacral segments is reported. The patient presented with systemic tuberculosis symptoms and signs, and progressive paraparesis. RESULTS: The patient was treated with antituberculous drug therapy and was operated twice for thoracolumbar and cervical spinal lesions. She made an excellent neurological recovery. CONCLUSIONS: In the patients with non-contiguous spinal involvement, there is a high percentage of requirement of surgical treatment. This may be due to "fulminant" behaviour of the disease in these patients. Early surgical treatment of the cases with large abscesses and systemic tuberculosis may provide early improvement, and must probably be the first treatment modality after general support to the patient.

Aggressive postoperative lumbar fibromatosis after the placement of instrumentation for treatment of spondylolisthesis. Case report.

This 50-year-old woman presented with a paravertebral lumbar fibromatosis (desmoid tumor) after undergoing the placement of instrumentation for lumbar spondylolisthesis. The tumor developed just cranial to the previous skin incision. Fibromatoses, or desmoid tumors, are uncommon infiltrative lesions that affect musculoaponeurotic structures, most often of the trunk and limbs. They are known to occur in association with surgery-related scars or implants and only rare examples appear in the neurosurgical and spine-related literature. In cases involving well-defined tumors in which radical resection is possible, surgery is the treatment of choice; however, the recurrence rate is high. The patient in the present case was followed for 14 months postoperatively and did not undergo radiotherapy; there was no recurrence. Although rare, this distinctive tumor should be considered in the differential diagnosis of postoperative paravertebral bulgings after neurosurgical and spinal operations.

Thoracic and lumbar tuberculous spondylitis treated by posterior debridement, graft placement, and instrumentation: a retrospective analysis in 19 cases.

OBJECT: Surgical treatment of thoracic and lumbar tuberculous spondylitis is controversial. An anterior approach is usually recommended. The aim of the present study was to assess the efficacy of posterior debridement and the placement of posterior instrumentation for the treatment of patients with thoracic and lumbar tuberculous spondylitis. METHODS: Nineteen patients with thoracic and lumbar tuberculous spondylitis underwent single-stage posterior decompression and debridement as well as the placement of posterior interbody grafts if necessary, instrumentation and posterior or posterolateral grafts. No postoperative neurological deterioration was noted. One patient died of myocardial infarction on Day 10. The mean follow-up duration, excluding the one death, was 52.7 months (range 16-125 months). In a 70-year-old patient, a single pedicle screw broke after 3 months. All patients were in better neurological condition after surgery and at the last follow-up examination. Neurological deficits were present in only two patients at the last follow up (one American Spinal Injury Association Grade B and one Grade C deficit preoperatively). Three other patients suffered intermittent back or low-back pain. The mean angulation measured in 13 patients with kyphotic deformity was 18.2 degrees (range 5-42 degrees) preoperatively; this was reduced to 17.3 degrees (range 0-42 degrees) after surgery. There was a 2.8 degrees loss of correction (range 2-5 degrees) after 44.3 months (16-64 months). Kyphosis did not progress beyond 15 months in any patient. CONCLUSIONS: A posterior approach in combination with internal fixation and posterior or posterolateral fusion (with or without placement of posterior interbody grafts) may be sufficient for the debridement of the infection and to allow spinal stabilization in patients with thoracic and lumbar tuberculous spondylitis. This procedure is associated with easy access to the spinal canal for neural decompression, prevention of loss of corrected vertebral alignment in the long term, and facilitation of early mobilization.

A solitary calvarial lytic lesion with typical histopathological findings of juvenile hyaline fibromatosis.

Juvenile hyaline fibromatosis (JHF) is a rare systemic disease characterized by papulonodular skin lesions, gingival hyperplasia, joint contractures, and osteolytic lesions on long bones and the skull. It has recently been reported that the disease is caused by mutations in the gene encoding capillary morphogenesis protein-2 (CMG-2). To date, fewer than 60 cases have been published in the literature. Partial disease expression is common, but no cases featuring a solitary calvarial lesion have been reported. The authors discuss this 4-year-old boy with a solitary calvarial osteolytic lesion whose histopathological examination exhibited findings characteristic of JHF. Mutational analysis, however, revealed that there were no mutations in the CMG-2 gene. Two years after surgery, he was free of any complaints as well as gingival hyperplasia, joint contractures, and new skull or skin lesions. This patient's condition may represent clinical or genetic heterogeneity associated with JHF. Whether solitary lesions mimicking JHF can arise from somatic mutation of the CMG-2 gene remains to be proven.

Paradoxical intracranial tuberculoma requiring surgical treatment.

Paradoxical enlargement of intracranial tuberculomas or development of new ones during adequate antituberculous chemotherapy is an uncommon event. Treatment of such cases is controversial. Steroid therapy is usually advocated without change in the antituberculous drug program. However, in some patients whose lesions fail medical treatment, or who have superficially located large lesions, surgical therapy may be required. A 15-year-old girl with pulmonary miliary tuberculosis, tuberculous meningitis and multiple intracranial tuberculomas is presented. While her pulmonary lesions and intracranial tuberculomas except one were healed with chemotherapy, one tuberculoma increased in size 1 month after starting chemotherapy. She was followed with dexamethasone treatment in addition to antituberculous therapy for 2 months. Then, the lesion was removed because it had increased in size in spite of appropriate chemotherapy.

Polyostotic monosystemic calvarial and spinal langerhans' cell histiocytosis treated by surgery and chemotherapy.

There are several treatment modalities for Langerhans' cell histiocytosis (LCH) of bone, formerly usually referred to as eosinophilic granuloma, including surgery, radiotherapy and chemotherapy. In spinal lesions, surgery is not recommended generally, because of the potential for reconstitution of vertebral height. We report a case of multifocal monosystemic LCH treated by surgery and chemotherapy for calvarial and spinal lesions. A 7-year-old boy was initially operated for an occipital monostotic LCH of bone. Then, he was treated by surgery for cervical and thoracic lesions, and by chemotherapy with vinblastine. The patient had no complaints at the 24-month follow-up. Although surgery is not recommended for spinal lesions in children with LCH of bone, short segmental fusion and internal fixation may be a suitable choice for selected cases, especially in children who are not likely to comply with activity restrictions and in those who it is difficult to follow up. The treatment must be planned according to the characteristics of the patient.